​Encephalocele

Diagnosing an Encephalocele

Ultrasound images of the fetus can reveal the presence of a herniated, fluid-filled sac outside the skull.

Encephaloceles that go undetected during gestation usually are diagnosed at birth by observation of the deformity.

Disorders associated with an encephalocele

Other developmental disorders usually accompany encephalocele. Once diagnosed, doctors will search for these other conditions or abnormalities, such as:

• Hydrocephalus (excessive accumulation of cerebrospinal fluid in the ventricles of the brain)
• Microcephaly (abnormally small head)
• Paralysis

In rare cases, a small encephalocele in the nasal cavity can remain undetected for years, even into adulthood.

An MRI is the preferred test to identify the contents of the sac. This is essential for determining the best treatment for an encephalocele.

Encephalocele Treatment

Surgery to remove the herniated sac and repair the opening in the skull is the only encephalocele treatment.

If the encephalocele is covered by normal skin rather than a thin membrane, surgery may be postponed until the child is older.

In most cases, any neural tissue contained in the sac is abnormal and surgeons will remove it. However, if healthy neural tissue is present in the sac, one of the goals of surgery is to preserve it.

The surgical approach depends on the location and contents of the encephalocele. If the sac does not contain vital nervous tissue, surgeons simply remove the encephalocele and repair the opening in the skull.

Minimally invasive surgery

Encephaloceles of the skull base may be approached directly using the Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the encephalocele through the nose and nasal cavities.

EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.